From: TSS(wt-d6-186.wt.net)Subject: The Contagion of FearDate: October 25, 2004 at 12:55 pm PST

The Contagion of Fear

October 25, 2004 By MARC SANTORA

The disease's fatal grip on the brain seems to come out ofnowhere, afflicting an otherwise healthy person and turninghim into an incoherent muddle. In Ulster County in upstateNew York, that was how death came to at least two people inthe last year. The rare brain malady calledCreutzfeldt-Jakob disease caused the death of one residentin November 2003 and of a second this fall.

About one in a million people worldwide die from themysterious disease each year, and there is no medicalexplanation for why the disease afflicts humans in its mostcommon form.

There has been a recent buzz in Ulster County about thestrange illness, but only within the small circles of thevictims' families.

Then the community discovered that a 60-year-old woman haddied in August from an illness that seemed similar toCreutzfeldt-Jakob disease. The woman's family did not allowan autopsy, so it cannot be determined if she had thedisease. But after comparing notes, people found out thatanother person in nearby Dutchess County died of thedisease this year.

There have been other unusual deaths, including that ofanother woman in Ulster County, Colleen Staccio, 46, whodied in August from a condition her doctors initially saidwas Creutzfeldt-Jakob disease but whose autopsy showedotherwise, and that of Richard Tobey, 59, who died of thedisease two weeks ago.

The community's response has gone from curious to downrightscared.

State health inspectors investigating the deaths said theycould not discuss specifics of the cases, citing privacyconcerns. They did say that they saw no risk to publichealth.

That has done little to ease public concern.

A state health investigator who looked into the deathsagreed to speak with The New York Times to ease publicconcern but insisted on anonymity. He said that, at most,the deaths represented a "statistical anomaly."

The investigator said that the woman whose family refusedan autopsy had been sick when she moved to Ulster County,that Creutzfeldt-Jakob disease had been ruled out in Ms.Staccio's case and, most important, that none of the deathswere the result of a form of Creutzfeldt-Jakob disease thatcan be caused by outside factors like infected meat.

That form of the disease, called variant Creutzfeldt-Jakobbut more commonly referred to as mad cow disease, is whatbrought the malady to public attention in the late 1990's.

Many people ate cattle in England infected with bovinespongiform encephalopathy, and as a result came down withthe variant form of the disease. The cases caused a panicaround the world.

In the United States, there has only been one documentedcase of someone dying from variant Creutzfeldt-Jakob, awoman in Florida who had lived in England.

Despite the fact that there is little evidence of that formof the disease here, the fear in Ulster County is that someoutside factor is causing the deaths. That is not the case,state health officials said. They confirmed the deaths inUlster and Dutchess Counties and another case in nearbyOrange County.

But, they noted, every year in New York State, around 20people die from what is called sporadic Creutzfeldt-Jakobdisease. The deaths in upstate New York are not consideredout of the ordinary, except that people are talking aboutthem.

Because so little is known about the disease, the fact thatanswers were hard to come by only added to the confusion.

"They're saying it's not a big deal, but I don't buy thatat all," said Jane Scordalakis, who was window-shoppinglast week in Kingston, N.Y., where several of the victimslived and were initially treated.

A staid, small city, Kingston is a community whereindividuals often cross paths. "They said there were twopeople in the hospital at the same time with this disease,but that there is no correlation," Ms. Scordalakis said."How do they know that there is no correlation?"

Creutzfeldt-Jakob disease is caused by a misformed protein- called a prion - in the brain. According to the WorldHealth Organization, roughly 85 percent of the cases of thedisease around the globe are sporadic, meaning people whoget the illness, typically those over 65, are exposed to noknown risk factors.

The disease can lie dormant for years, even decades, anddoctors do not know what sets it off. There are only ahandful of known prion diseases that afflict humans, andall are fatal. Creutzfeldt-Jakob disease has been thesubject of serious medical study for the past 10 to 20years, but the cases in Ulster County are typical of theway the disease works.

Mr. Tobey was in relatively good health when he got sickthis past summer. At first, his daughter, Stacey Tobey,noticed only little changes - the world seemed to begetting fuzzier for her father.

"Basically, he progressed to where he couldn't eat,couldn't swallow, couldn't speak," Ms. Tobey said. Doctorswere puzzled by his rapid deterioration. At first theythought it might have been related to his sleep apnea, thenthey thought he must have suffered a stroke.

In fact, the state health inspector said, reports ofsymptoms that seem to suggest Creutzfeldt-Jakob diseaseoften turn out to be caused by strokes or the rapid onsetof Alzheimer's disease.

A biopsy confirmed that Mr. Tobey was indeed suffering fromCreutzfeldt-Jakob disease. He died less than three monthsafter he first started showing symptoms.

Richard Joseph Da Silva, 58, from Highland Mills in OrangeCounty, suffered in a similar manner before he died in Mayof what his wife, Ann Marie Da Silva, said was listed onhis death certificate as Creutzfeldt-Jakob disease. Mrs. DaSilva, contacted The New York Times after reading about thecases in Ulster County. She was upset that the state healthofficials had not gotten in touch with her to find out moreabout Mr. Da Silva's death.

One senior state health official, who said he did not wantto be named because of patient privacy concerns, saidinvestigators rarely contact families in deaths involvingdementia, an effect of the disease. A New York State lawrequires health inspectors to examine every death caused bydementia - which means they examine hundreds of cases ayear. In the Health Department's division of chronicdisease, several people are devoted solely to this task.

When the department investigates deaths believed to havebeen caused by Creutzfeldt-Jakob disease, it firstdetermines the actual cause of death and then decides ifthere is a cluster of related deaths or just a geographiccoincidence. More often than not, officials said, it is acoincidence.

For instance, in New Jersey, a series of deaths fromCreutzfeldt-Jakob disease in the Cherry Hill area in recentyears caused a stir after people started looking at thedeath certificates and saw all sorts of perceived patterns.

Dr. Clifton R. Lacy, the New Jersey health commissioner,said that a lengthy investigation found nothing out of theordinary, but that did not stop the public from worrying.

The New York health investigator who looked into the deathsin Ulster County said, "Often the geographic patterns thatyou see have no relevance."

But Mrs. Da Silva said, "I am just feeling veryfrustrated."

She said she had read extensively about the disease and waslooking for any possible clues. For example, she said thather husband had served in the Vietnam War. "He saw theworst of the worst," Mrs. Da Silva said. "He did deal withopen brains, he was holding people's body parts."

Until the weeks before his death, Mr. Da Silva had been ingood health and had worked for years as a dean at RutgersUniversity, Mrs. Da Silva said. Then, last spring, hesuddenly changed.

"He started shaking," Mrs. Da Silva said. "He just becamevery frightened and he kept saying, 'There is like a glazeover everything I am seeing.' ''

Mr. Da Silva died a few weeks later, on May 13, 2004.

Florence Kranitz, the president of the Creutzfeldt-JakobDisease Foundation in Ohio, said the frustration ofrelatives of those who die from the disease wasunderstandable. "Sporadic C.J.D. is a very enigmatic formof this disease and it seems to happen for no apparentreason," she said. "This is a scary disease. It's one ofthe most horrible on the face of the earth."

In the Health Department's division of chronic disease, >>several people are devoted solely to this task. >

THE EPIDEMIOLOGY OF CJD RG WILL 1984 (182 PAGES)

snip...

One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...

snip...

http://www.bseinquiry.gov.uk/files/mb/m26/tab01.pdf

>Florence Kranitz, the president of the Creutzfeldt-Jakob Disease >Foundation in Ohio, said the frustration of relatives of those who >die from the disease was understandable. "Sporadic C.J.D. is a very >enigmatic form of this disease and it seems to happen for no apparent >reason," she said. >

RIGHT! and as long as this foundation and others continue to ignore thescience, the agent will always be sporadic without any route and source.as soon as the cdc came through the back door of the CJD Foundationyou could see the writing on the wall, all sporadic. what better way thanto have all diangosed cjd be sporadic, than to have one central surveillanceunit over riding any atyical TSEs in humans diagnosed by any other doctor.how many times has this happened already? yep, just my 2 bits worth ...the bse/nvCJD only theory should be trashed once and for all...

In an experimental study of the transmissibility of BSE to the pig,seven of 10 pigs, infected at 1-2 weeks of age by multiple-routeparenteral inoculation with a homogenate of bovine brain fromnatural BSE cases developed lesions typical of spongiformencephalopathy...

THE recent discoveries of previously unidentified strains ofScrapie such as 221C44 and the Nor9845;

FULL TEXT APPRX. 91 PAGES

UK Strategy for Research andDevelopment on Human and AnimalHealth Aspects of TransmissibleSpongiform Encephalopathies

2004-2007

http://www.mrc.ac.uk/pdf-uk_strategy_v5.2.pdf

WHEN in fact, the findings from Marsh and the findings atMISSION, TEXAS support even further evidence that thereare further strains of TSE in the USA besides that oneaccidentally documented BSE case in Washington onDec. 23, 2003;

In Confidence - Perceptions of unconventional slow virus diseases of animals in the USA - Report of a visit to the USA - April-May 1989 - G A HWells [head of England's main veterinary lab]

THE exact same thing that happened in the uk is happening in the usa,and it's called 'COVER-UP' 'PROTECT THE INDUSTRY AT ALL COST'.

CJD FARMERS WIFE 1989

http://www.bseinquiry.gov.uk/files/yb/1989/10/13007001.pdf

http://www.bseinquiry.gov.uk/files/yb/1989/10/13003001.pdf

20 year old died from sCJD in USA in 1980 and a 16 yearold in 1981. A 19 year old died from sCJD inFrance in 1985. There is no evidence of an iatrogeniccause for those cases....

http://www.bseinquiry.gov.uk/files/yb/1995/10/04004001.pdf

cover-up of 4th farm worker ???

http://www.bseinquiry.gov.uk/files/yb/1995/10/23006001.pdf

http://www.bseinquiry.gov.uk/files/yb/1995/10/20006001.pdf

CONFIRMATION OF CJD IN FOURTH FARMER

http://www.bseinquiry.gov.uk/files/yb/1995/11/03008001.pdf

now story changes from;

SEAC concluded that, if the fourth case were confirmed, it would beworrying, especially as all four farmers with CJD would have had BSEcases on their farms.

to;

This is not unexpected...

was another farmer expected?

http://www.bseinquiry.gov.uk/files/yb/1995/11/13010001.pdf

4th farmer, and 1st teenager

http://www.bseinquiry.gov.uk/files/yb/1996/02/27003001.pdf

2. snip...Over a 5 year period, which is the time period on which the advicefrom Professor Smith and Dr. Gore was based, and assuming apopulation of 120,000 dairy farm workers, and an annual incidenceof 1 per million cases of CJD in the general population, aDAIRY FARM WORKER IS 5 TIMES MORE LIKELY THANan individual in the general population to develop CJD. Using theactual current annual incidence of CJD in the UK of 0.7 permillion, this figure becomes 7.5 TIMES.

3. You will recall that the advice provided by Professor Smith in1993 and by Dr. Gore this month used the sub-population of dairyfarm workers who had had a case of BSE on their farms -63,000, which is approximately half the number of dairy farmworkers - as a denominator. If the above sums are repeated usingthis denominator population, taking an annual incidence in the generalpopulation of 1 per million the observed rate in this sub-populationis 10 TIMES, and taking an annual incidence of 0.7 per million,IT IS 15 TIMES (THE ''WORST CASE'' SCENARIO) thanthat in the general population...

http://www.bseinquiry.gov.uk/files/yb/1995/01/31004001.pdf

AND IT'S CALLED 'THE USA ENHANCED COVER-UP OF BSE' ;HERE IS THE TEXAS MAD COW THAT WENT TO THE RENDER WITHOUT BEING TESTED AND OTHER MULTIPLE FLAWS IN THE SYSTEM;

July 13, 2004

IG Audit Finds Multiple Flaws in Mad Cow Surveillance PlanRep. Waxman raises questions about the effectiveness and credibility ofUSDA's response to mad cow disease, citing an audit by the USDAInspector General that finds systemic deficiencies in the Department'ssurveillance plan and new evidence that USDA misled the public in thewake of the detection of an infected cow in Washington State.

Failure To Test Staggering Cow May Reflect Wider ProblemsRep. Waxman raises concerns that the recent failure of USDA to test animpaired cow for BSE may not be an isolated incident, citing the failureof USDA to monitor whether cows condemned for central nervous systemsymptoms are actually tested for mad cow disease.

On Friday, April 30 th , the Food and Drug Administration learned that a cow with central nervous system symptoms had been killed and shipped to a processor for rendering into animal protein for use in animal feed.

FDA, which is responsible for the safety of animal feed, immediately began an investigation. On Friday and throughout the weekend, FDA investigators inspected the slaughterhouse, the rendering facility, the farm where the animal came from, and the processor that initially received the cow from the slaughterhouse.

FDA's investigation showed that the animal in question had already been rendered into "meat and bone meal" (a type of protein animal feed). Over the weekend FDA was able to track down all the implicated material. That material is being held by the firm, which is cooperating fully with FDA.

Cattle with central nervous system symptoms are of particular interest because cattle with bovine spongiform encephalopathy or BSE, also known as "mad cow disease," can exhibit such symptoms. In this case, there is no way now to test for BSE. But even if the cow had BSE, FDA's animal feed rule would prohibit the feeding of its rendered protein to other ruminant animals (e.g., cows, goats, sheep, bison).

FDA is sending a letter to the firm summarizing its findings and informing the firm that FDA will not object to use of this material in swine feed only. If it is not used in swine feed, this material will be destroyed. Pigs have been shown not to be susceptible to BSE. If the firm agrees to use the material for swine feed only, FDA will track the material all the way through the supply chain from the processor to the farm to ensure that the feed is properly monitored and used only as feed for pigs.

To protect the U.S. against BSE, FDA works to keep certain mammalian protein out of animal feed for cattle and other ruminant animals. FDA established its animal feed rule in 1997 after the BSE epidemic in the U.K. showed that the disease spreads by feeding infected ruminant protein to cattle.

Under the current regulation, the material from this Texas cow is not allowed in feed for cattle or other ruminant animals. FDA's action specifying that the material go only into swine feed means also that it will not be fed to poultry.

FDA is committed to protecting the U.S. from BSE and collaborates closely with the U.S. Department of Agriculture on all BSE issues. The animal feed rule provides crucial protection against the spread of BSE, but it is only one of several such firewalls. FDA will soon be improving the animal feed rule, to make this strong system even stronger.

WASHINGTON, Aug. 11 (UPI) -- The U.S. Department of Agriculture failedto test for mad cow disease or collect the correct portion of the brainon nearly 500 suspect cows over the past two years -- including some incategories considered most likely to be infected -- according to agencyrecords obtained by United Press International.

The testing problems mean it may never be known with certainty whetherthese animals were infected with the deadly disease. Departmentofficials said these animals were not included in the agency's finaltally of mad cow tests, but the records, obtained by UPI under theFreedom of Information Act, indicate at least some of them were counted...